• Jin Young Kim, Hye Jeong Oh, Yuntae Kim, and Jin Myoung Seok.
• Department of Physical Medicine and Rehabilitation, Soonchunhyang University Hospital Bucheon, Soonchunhyang University College of Medicine, Bucheon.
• Medicine (Baltimore). 2021 Apr 23; 100 (16): e25580e25580.

RationaleNeuromyelitis optica spectrum disorder (NMOSD) is a severe inflammatory disorder of the central nervous system with an autoantibody against aquaporin-4 protein (AQP4), and amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. We report a female patient with ALS who had asymptomatic AQP4 antibody at the diagnosis of ALS, and NMOSD occurred 4 years later after the diagnosis of ALS.Patient ConcernsShe was already bedridden and had tracheostomy because of ALS which was diagnosed at her age of 55. At the time of her ALS diagnosis, she had no brain or spinal cord lesions, but was seropositive for AQP4 antibody. At her age of 59, new-onset complete paralysis of all extremities and severe pain on the posterior neck and both shoulders occurred and visited the hospital.DiagnosisLongitudinally extensive transverse myelitis was diagnosed, which was the onset attack of seropositive NMOSD. The diagnosis was confirmed based on the international consensus diagnostic criteria for NMOSD with MR imaging, cerebrospinal fluid exam and laboratory work-ups with AQP4 antibody test.InterventionsHigh dose methylprednisolone was administered for 5 days. Plasma exchange as a further treatment was recommended, but she and her family refused.OutcomesHer pain was relieved after steroid treatment, but there was no improvement of her leg weakness.LessonsThis case is a rare combination of neuroinflammatory and neurodegenerative diseases. Considering the alterations of blood-brain barrier along with the progression of ALS, it highlights that the consequence of ALS pathogenesis might affect the development of NMOSD. And the careful follow-up is recommended even in patients with profound weakness, especially if those who were at risk of developing certain neurological disorders.Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.

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